Surgical Debulking of Persistent Orofacial Swelling in a Patient with Melkersson–Rosenthal Syndrome

Abstract

Summary:. Melkersson–Rosenthal syndrome (MRS) is a rare neuro-mucocutaneous granulomatous disorder that is likely underdiagnosed due to its variability on presentation. Few patients present with the characteristic triad of orofacial swelling, recurrent facial palsy, and lingua plicata; in fact, most patients present with only one or two of the symptoms. Additionally, patients are evaluated by a variety of specialists, including ophthalmologists, otolaryngologists, dermatologists, and plastic surgeons, depending on their particular symptom. There is no consensus standard of care for MRS due to a limited understanding of the disease. We describe a case of a patient with a 5-year history of persistent orofacial edema in the setting of Melkersson–Rosenthal syndrome refractory to medical management, who was treated with surgical debulking of the upper lip. We encourage increased clinical suspicion for Melkersson–Rosenthal syndrome when evaluating persistent orofacial edema in the plastic surgery clinic and highlight the importance of obtaining a full patient history. Surgical debulking can correct for aesthetic deformity while sparing oral function.